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Jaap Bethlem, MD; Willem Frans Arts, MD; Koert P. Dingemans, MD

Arch Neurol. 1978;35(9):555-566.

Abstract
• Twelve patients suffering from congenital neuromuscular disorders showed at least two of the following structural changes in their muscle biopsy specimens: rods, cores, miniature cores (M lesions), and focal loss of cross-striations (F lesions).

There appeared to be no essential differences between M and F lesions except for the presence of vesicular nuclei in and around the latter.

A striking finding was the presence of adjacent M lesions, F lesions, or both in two or several adjoining muscle fibers. Serial sections often showed a definite relationship of the lesions with blood vessels. The finding of rods, cores, and M and F lesions, not only in one biopsy specimen but also in one muscle fiber, raised the intriguing possibility of common pathogenic mechanisms producing these lesions.

Author Affiliations
From the Muscle Research Center of the Neurological Department (Drs Bethlem and Arts) and the Department of Electron Microscopy of the Pathology Laboratory (Dr Dingemans), University of Amsterdam, The Netherlands.

Footnotes
Accepted for publication Feb 20, 1978.

Read before the 11th World Congress of Neurology, Amsterdam, Sept 11, 1977.

Reprint requests to Neurological Department, Academic Hospital of the University of Amsterdam, Wilhelmina Gasthuis, le Helmerstraat 104, Amsterdam, The Netherlands (Dr Bethlem).

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